DIFFICULT CASES: Severely Subluxated Pediatric Cataract in an Eye With Aniridia and Glaucoma
My most difficult cataract case involved a host of complexities and the need for a solution that would last for decades.
HISTORY
I had gone to Mumbai, India, to deliver a lecture and
perform live surgery of a glued IOL, a technique I began
using in 2007.1-4 After my lecture, an ophthalmologist
from that region approached me and said he had a
pediatric patient with a badly subluxated cataract,
which was complicated by aniridia and glaucoma. He
wanted to refer the case to me. I agreed to see the
patient. On my flight home, I wondered how I might
approach such a complex case.
The 8-year-old child and his parents came to see me in Chennai, India. When I examined the child, I was shocked. The cataract was badly subluxated, and aniridia was evident (Figure 1). The IOP was 35 mm Hg. The boy’s other eye had the same problems. After a thorough examination, I prescribed antiglaucoma medications to reduce the patient’s IOP.
OPTIONS
Obviously, I could not leave the child as he was. His
vision was counts fingers at 1 meter, and he had glaucoma.
I could perform glaucoma surgery and remove the
cataract after explaining to his parents that he would
have to wear thick, aphakic glasses for the rest of his life.
This was not an ideal solution.
I did not want to suture an IOL to the sclera in the eye of a child. My concerns about scleral fixation were amplified, because the sutures would have to hold for at least 60 years. Neither an iris-fixated IOL (an iris claw lens or a PCIOL sutured to the iris) nor an ACIOL was an option because of the child’s aniridia.
I could remove the cataract and place a Cionni Ring for Sclera Fixation (Morcher GmbH, Stuttgart, Germany; distributed in the United States by FCI Ophthalmics, Marshfield Hills, MA). Then, I could perform a trabeculectomy and try to implant a PCIOL in the bag. The massive subluxation of the crystalline lenses eliminated this option as well as the use of an Ahmed Capsular Tension Segment (Morcher GmbH, Stuttgart, Germany; distributed in the United States by FCI Ophthalmics, Inc., Marshfield Hills, MA) or the AssiAnchor (not available in the United States; Hanita Lenses, Kibbutz Hanita, Israel).
None of the strategies I considered would address the patient’s aniridia or the glare and discomfort the child would experience postoperatively.
SURGICAL PLAN
I explained the limited options to the patient’s parents.
I told them I planned to address their son’s glaucoma
surgically. I would remove the cataract and perform
a vitrectomy (Figure 2A and B). Finally, in an effort to
address the boy’s aphakia and aniridia, I would fixate
with glue an aniridic IOL to the sclera.
I chose a PMMA aniridia IOL (Figure 2C), specifically the OV lens (model ANI5; Intra Ocular Care, Gujarat, India). The overall diameter of the implant is about 12.75 mm. The optic has a central clear zone of about 5 mm with a peripheral opaque or pigmented annulus of approximately 9.5 mm. The haptics are also made of PMMA.
SURGICAL COURSE
I created two partial-thickness scleral flaps of about 2.5 X
3.0 mm that were exactly 180° apart diagonally. I used an
infusion cannula for fluid, but the procedure can also be
performed with a 23-gauge sutureless trocar infusion cannula.
I created a third scleral flap for the
trabeculectomy. Next, I made a scleral
tunnel incision between the flaps and
completed the lensectomy to remove
the subluxated cataractous lens (Figure
2A and B) with the vitrectomy cutter. I
performed an anterior vitrectomy to
relieve any traction on the vitreous. I
created two straight sclerotomies with
a 20-gauge needle under the existing
scleral flaps and used a sharp keratome
to enlarge the scleral tunnel incision.
With a McPherson forceps, I introduced the PMMA aniridia implant through the limbal incision. I passed an end-gripping 23-gauge microcapsulorhexis forceps (MicroSurgical Technology, Redmond, WA) through one of the sclerotomies to hold the tip of the haptic. I then externalized both of the haptics under each of the scleral flaps. I closed the scleral tunnel with 10–0 monofilament nylon sutures. Next, I made a scleral tunnel with a 26-gauge needle at the point of the haptic’s externalization and tucked it into the intralamellar scleral tunnel (Figure 2D). After closing both of the scleral flaps with fibrin glue (Tisseel glue; Baxter Healthcare Corporation, Glendale, CA), I performed the trabeculectomy. I then sutured the trabeculectomy scleral flap.
I removed the infusion cannula and apposed the conjunctiva with fibrin glue.
OUTCOME
The patient did very well postoperatively. The IOL
remained in place, and the IOP was well controlled at
18 mm Hg. Six months postoperatively, I saw the child
and the happiness on his face and his parents’, too.
The boy was now able to go to school. They were all
comparing the vision of the operated eye and unoperated
eye and could not believe the difference. The parents
wanted me to proceed with surgery on their son’s
second eye immediately.
GLUED IRIS PROSTHESIS:
A NEW TREATMENT FOR ANIRIDIA
An intact iris diaphragm is essential, because it
decreases spherical and chromatic aberrations arising
from the lens. The symptoms of aniridia range from
decreased vision to optical disturbances such as glare and
photophobia due to excess light. Eyelid surgeries, colored
contact lenses, scleral suture-fixated prostheses, foldable artificial irides, and corneal tattooing are widely used to
improve patients’ symptoms. Contact lenses must be
removed periodically, and a scleral suture-fixated IOL can
be associated with postoperative hypotony, inflammation,
and cystoid macular edema. A glued iris prosthesis
avoids these problems.1-4
The procedure can be performed on eyes with traumatized corneas that have any grade of scarring, a contraindication for contact lenses. Moreover, the incidence of uveitis-glaucoma-hyphema syndrome is far lower than with a scleral suture-fixated IOL, a sulcusfixated prosthesis, or an artificial iris.4
The placement of a glued iris prosthesis may be combined with other surgeries such as penetrating keratoplasty, trabeculectomy, and pars plana vitrectomy.
I wish I could say I had invented the glued IOL technique, but I must credit divine inspiration. My hope is that this technique will help patients such as the one described herein see better.
Section editor David F. Chang, MD, is a clinical professor at the University of California, San Francisco. Dr. Chang may be reached at (650) 948-9123; dceye@earthlink.net
Amar Agarwal, MS, FRCS, FRCOphth, is the director of Dr. Agarwal’s Group of Eye Hospitals in Chennai, India. He acknowledged no financial interest in the products or companies mentioned herein. Professor Agarwal may be reached at +91 44 2811 6233; dragarwal@vsnl.com.
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